The hot cross bun sign in corticobasal degeneration.
Parkinsonian disorders
dementia
magnetic resonance imaging
multiple system atrophy
neuropathology
Journal
Neuropathology : official journal of the Japanese Society of Neuropathology
ISSN: 1440-1789
Titre abrégé: Neuropathology
Pays: Australia
ID NLM: 9606526
Informations de publication
Date de publication:
Oct 2021
Oct 2021
Historique:
revised:
25
04
2021
received:
20
03
2021
accepted:
25
04
2021
pubmed:
29
7
2021
medline:
14
1
2022
entrez:
28
7
2021
Statut:
ppublish
Résumé
The hot cross bun (HCB) sign encompasses a cross-shaped hyperintensity area in the pons on axial T2-weighted magnetic resonance imaging (MRI). The HCB sign is characteristic of multiple system atrophy (MSA) and has occasionally been observed in other neurological disorders. Here, we report an autopsied case of corticobasal degeneration (CBD) that showed the HCB sign. A female patient presented with progressive gait disturbance and cognitive impairment at the age of 60 years. A neurological examination revealed dysarthria, muscle rigidity of the limbs, akinesia, truncal ataxia, urinary incontinence, and dementia. The HCB sign was observed on a brain MRI at the age of 65 years, and a clinical diagnosis of possible MSA was made. She died of pneumonia at the age of 67 years. A postmortem observation, provided neuropathological findings characteristic of CBD, including the presence of astrocytic plaques, pretangles, neuropil threads, and ballooned neurons in association with four-repeat-tau aggregation. Interestingly, the pons displayed severe neuronal loss and astrogliosis that were prominent in the pontine and raphe nuclei. Myelin sheath depletion was prominent in the transverse fibers of the pontine base and the myelinated fibers of the pontine tegmentum in contrast to relative sparing of the pontine corticospinal tract and medial lemniscus. The cerebellar dentate nucleus exhibited neuronal loss and grumose degeneration. Western blot analysis of sarkosyl-insoluble fractions from brain tissue lysates using an anti-phosphorylated tau antibody identified immunoreactive signal bands in approximately 37-40, 43, 64, and 68 kDa, consistent with CBD. Genetic analysis did not reveal any known pathogenic mutations in the microtubule-associated protein tau gene (MAPT). Our case was characterized by the HCB sign and concordant neuropathological changes in the pons. CBD should be considered an underlying pathology of the HCB sign, even though the pontocerebellar changes would be unusual in CBD cases.
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
376-380Subventions
Organisme : Grants-in-Aid from the Research Committee of CNS Degenerative Diseases, Research on Policy Planning and Evaluation for Rare and Intractable Diseases, Health, Labour, and Welfare Sciences Research Grants, the Ministry of Health, Labour and Welfare, Japan
Organisme : Intramural Research Grant for Neurological and Psychiatric Disorders of NCNP
ID : 30-8
Organisme : Japan Agency for Medical Research and Development
ID : JP20ek0109391
Organisme : Japan Agency for Medical Research and Development
ID : JP20ek0109392
Organisme : JSPS KAKENHI
ID : JP20K16586
Informations de copyright
© 2021 Japanese Society of Neuropathology.
Références
Schrag A, Kingsley D, Phatouros C et al. Clinical usefulness of magnetic resonance imaging in multiple system atrophy. J Neurol Neurosurg Psychiatry 1998; 65: 65-71.
Shrivastava A. The hot cross bun sign. Radiology 2007; 245: 606-607.
Zhu SZ, Li HL, Deng B et al. Various diseases and clinical heterogeneity are associated with “hot cross bun”. Front Aging Neurosci 2020; 12: 592212.
Kim KW, Lee DY, Jhoo JH et al. Diagnostic accuracy of mini-mental status examination and revised hasegawa dementia scale for Alzheimer's disease. Dement Geriatr Cogn Disord 2005; 19: 324-330.
Gilman S, Wenning GK, Low PA et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology 2008; 71: 670-676.
Braak H, Alafuzoff I, Arzberger T, Kretzschmar H, Del Tredici K. Staging of Alzheimer disease-associated neurofibrillary pathology using paraffin sections and immunocytochemistry. Acta Neuropathol 2006; 112: 389-404.
Thal DR, Rub U, Orantes M, Braak H. Phases of A beta-deposition in the human brain and its relevance for the development of AD. Neurology 2002; 58: 1791-1800.
Saito Y, Ruberu NN, Sawabe M et al. Staging of argyrophilic grains: An age-associated tauopathy. J Neuropathol Exp Neurol 2004; 63: 911-918.
Taniguchi-Watanabe S, Arai T, Kametani F et al. Biochemical classification of tauopathies by immunoblot, protein sequence and mass spectrometric analyses of sarkosyl-insoluble and trypsin-resistant tau. Acta Neuropathol 2016; 131: 267-280.
Yoshida M. Astrocytic inclusions in progressive supranuclear palsy and corticobasal degeneration. Neuropathology 2014; 34: 555-570.
Savoiardo M, Strada L, Girotti F et al. Olivopontocerebellar atrophy: MR diagnosis and relationship to multisystem atrophy. Radiology 1990; 174: 693-696.
Kouri N, Oshima K, Takahashi M et al. Corticobasal degeneration with olivopontocerebellar atrophy and TDP-43 pathology: An unusual clinicopathologic variant of CBD. Acta Neuropathol 2013; 125: 741-752.
Svenningsson P. Corticobasal degeneration: Advances in clinicopathology and biomarkers. Curr Opin Neurol 2019; 32: 597-603.
Fanciulli A, Wenning GK. Multiple-system atrophy. N Engl J Med 2015; 372: 249-263.
Yoshida M. Multiple system atrophy: Alpha-synuclein and neuronal degeneration. Neuropathology 2007; 27: 484-493.